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    OpenPayerBilling CodesCongenital malformations of the urinary systemICD-10-CM q61-cystic-kidney-disease
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    ICD-10-CM Code For Cystic Kidney Disease: Group Overview

    The International Classification of Diseases, Tenth Revision, Clinical Modification (ICD-10-CM) code group Q61 includes congenital cystic kidney disorders such as autosomal recessive and autosomal dominant polycystic kidney disease, multicystic dysplastic kidney, and other specified cystic malformations of the kidney that present at birth or early life. These ICD-10-CM diagnosis codes are used to document clinical scenarios ranging from isolated unilateral cystic dysplasia to bilateral hereditary polycystic disease with renal insufficiency, capturing disease severity, organ involvement, and associated complications. Accurate coding within the ICD-10-CM Q61 group supports proper claim adjudication, complete clinical documentation, and appropriate reimbursement.

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    ICD-10-CM Q61: Cystic Kidney Disease Overview

    The Q61 group covers congenital and hereditary cystic disorders of the kidneys, including autosomal and nonautosomal forms and localized cystic dysplasia. These conditions affect the renal parenchyma and collecting system with variable impact on kidney function and potential systemic complications. Accurate coding ensures clinical severity and organ involvement are captured for appropriate reimbursement and longitudinal tracking of congenital renal disease.

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    When to Use

    Use a code from this group when a congenital or hereditary cystic kidney condition has been diagnosed or when imaging/pathology documents cystic malformations of the renal parenchyma. Select the code that reflects laterality, specified disease subtype, and associated renal impairment when documented.

    ICD-10-CM Codes

    11 rows

    ICD-10-CM CodeDescription
    Q61.00Congenital renal cyst, unspecified
    Q61.01Congenital single renal cyst
    Q61.02Congenital multiple renal cysts
    Q61.11Cystic dilatation of collecting ducts
    Q61.19Other polycystic kidney, infantile type
    Q61.2Polycystic kidney, adult type
    Q61.3Polycystic kidney, unspecified
    Q61.4Renal dysplasia
    Q61.5Medullary cystic kidney
    Q61.8Other cystic kidney diseases
    Q61.9Cystic kidney disease, unspecified

    DRG Codes

    3 rows

    DRG CodeDescription
    698Other Kidney and Urinary Tract Diagnoses with MCC
    699Other Kidney and Urinary Tract Diagnoses with CC
    700Other Kidney and Urinary Tract Diagnoses without CC/MCC

    Typical Clinical Scenarios

    • An infant is born with palpable bilateral renal masses on newborn exam and prenatal ultrasound had shown enlarged, echogenic kidneys; nephrology evaluates the neonate for family history and renal function, and congenital bilateral cystic involvement consistent with autosomal recessive polycystic kidney disease is documented, prompting assignment of the congenital cystic kidney diagnosis. Typical codes: Q61.01, Q61.19
    • An adult with a known history of chronic hereditary cystic kidney disease attends routine nephrology follow-up for progressive hypertension and declining glomerular filtration rate; imaging confirms multiple cortical and medullary renal cysts consistent with autosomal dominant polycystic kidney involvement, so a chronic congenital cystic kidney code is used to denote the underlying congenital malformation as the cause of ongoing chronic kidney disease management. Typical codes: Q61.2, Q61.3
    • A pediatric patient presents to the emergency department with urinary tract infection and worsening flank pain; prior records note unilateral multicystic dysplastic kidney identified in infancy, and current imaging shows compensatory hypertrophy of the contralateral kidney with infected cystic changes in the dysplastic kidney, so the congenital cystic anomaly code for unilateral multicystic dysplastic kidney is assigned as a comorbid congenital condition influencing acute care. Typical codes: Q61.4, Q61.5

    Related Codes

    Q60-RENAL-AGENESIS-AND-REDUCTION-DEFECTS

    ICD-10-CM Code For Renal Agenesis And Other Reduction Defects Of Kidney: Group Overview

    Q62-CONGENITAL-OBSTRUCTIVE-DEFECTS-RENAL-PELVIS-URETER

    ICD-10-CM Code For Congenital Obstructive Defects Of Renal Pelvis And Congenital Malformations Of Ureter: Group Overview

    Q63-OTHER-CONGENITAL-MALFORMATIONS-OF-KIDNEY

    ICD-10-CM Code For Other Congenital Malformations Of Kidney: Group Overview

    Q64-OTHER-CONGENITAL-MALFORMATIONS-OF-URINARY-SYSTEM

    ICD-10-CM Code For Other Congenital Malformations Of Urinary System: Group Overview

    Frequently Asked Questions

    References

    1. 1.CPT® Codes and Descriptions. AAPC. Retrieved Aug 11, 2026.Maintains the CPT code set's official descriptions and guidelines, licensed from the American Medical Association, used as the definitional source for CPT codes on this page.
    2. 2.HCPCS Quarterly Update. Centers for Medicare & Medicaid Services (CMS). Retrieved Aug 11, 2026.CMS's quarterly release of HCPCS Level II codes and short/long descriptors, the source for HCPCS code definitions and update history.
    3. 3.ICD-10-CM Full Code List. Centers for Medicare & Medicaid Services (CMS). Retrieved Aug 11, 2026.CMS's complete ICD-10-CM diagnosis code list, used to cross-reference diagnosis codes associated with this procedure or service.
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